Membranoproliferative glomerulonephritis: A rare presentation of occult filariasis

Parasitic infections do not usually present with rapidly progressive renal failure but can provoke glomerular lesions which are mostly proliferative. In filarial infection, glomerular involvement is usually mild and transient, and presentation with renal failure is rare. We report occult filariasis presenting as rapidly progressive renal failure due to immune-complex mediated membranoproliferative glomerulonephritis. Our patient responded to treatment with diethylcarbamazine and a short course of steroid. This case highlights the importance of thorough workup to identify the cause and consideration of filariasis in an endemic area.

INF2 p.Arg214Cys mutation in a Chinese family with rapidly progressive renal failure and follow-up of renal transplantation: case report and literature review

Background Heterozygous mutations in the inverted formin 2 (INF2) gene are related to secondary focal segmental glomerulosclerosis (FSGS), a rare secondary disease associated with rapidly progressive renal failure. Case presentation We report a patient with familial autosomal INF2 mutation manifesting nephritic syndromes and elevated serum creatinine levels. Mutational analysis revealed an autosomal dominant (AD) inheritance pattern and a mutation in exon 4 (p.Arg214Cys) of INF2 as the likely cause, which has not been previously described in an Asian family. The patient progressed to end-stage renal disease (ESRD) and received hemodialysis. His mother had undergone renal transplant 3 years earlier, and his grandmother had carried the p.Arg214Cys mutation for more than 80 years without any sign of renal dysfunction. Conclusions This is the first report to identify an association between a familial autosomal dominant INF2 p.Arg214Cys mutation and rapidly progressive renal disease in an Asian family. INF2 mutation analysis should not be restricted to individuals without family history of FSGS, rather it should also be performed on individuals for whom drug-based therapies are not effective. In this case, kidney transplant is an effective alternative.

Otitis media with effusion as an initial manifestation of granulomatosis with polyangiitis

Granulomatosis with polyangiitis is a systemic vasculitis of unknown etiology, characterized by necrotizing granulomas. It is an autoimmune disease affecting small- and medium-sized vessels of upper and lower respiratory tract, kidneys, and other organs. We described a case of a patient with otitis media with effusion as the first manifestations of granulomatosis with polyangiitis. A 54-year-old female presented as an urgent case with history of a severe otalgia, hearing loss, vertigo, and fever. The patient was treated with diagnosis of otitis media with effusion and acute rhinosinusitis, but without significant success. She developed an acute kidney dysfunction as a sign of glomerulonephritis with rapidly progressive renal failure. Diagnosis of granulomatosis with polyangiitis was confirmed after the histopathological analysis of kidney tissue, not by analysis of middle ear and paranasal sinus mucosa specimens. The patient was treated according to generally accepted protocol, and over time, there was an almost complete recovery.

Liberation from Dialysis Dependence in a Patient with HIV-Associated Nephropathy (HIVAN) after Combined Antiretroviral Therapy (cART)

Prior to the advent of combined antiretroviral therapy (cART), human immunodeficiency virus-associated nephropathy (HIVAN) was inevitably associated with rapidly progressive renal failure and dialysis dependence. HIV-1 seropositive patients often met with untimely deaths due to complications of end-stage renal disease (ESRD), opportunistic infections, or other HIV-related end-organ failure. Although the association between cART and improved outcomes in HIVAN has been recognized for over 20 years, no randomized trials have specifically examined this effect to date. In terms of reversal of dialysis-dependent renal failure after cART initiation, only a handful of case reports exist. The authors report a case of a 44-year-old Latino male requiring thrice-weekly haemodialysis in the setting of biopsy-proven HIVAN who was able to stop dialysis in 7 months after being initiated on cART.

A study of clinicopathological features and outcomes of crescentic glomerulonephritis

Background: Crescentic Glomerulonephritis (CrGN) is characterized by rapidly progressive renal failure. Most of the literatures have defined >50% crescents in biopsy as CrGN. Only very few studies have included the presence of <50% crescents as CrGN.Methods: To assess the clinico-pathological features and outcome of CrGN with >10% crescents on renal biopsy and comparing them splitting our diagnosis into Immune Complex Mediated CrGN (ICCGN) and non-immune complex mediated CrGN (NICCGN) groups.Results: ICCGN was the commonest group. When compared to ICCGN group, NICCGN patients were older, anuric, had more glomerular necrosis and severe IFTA in biopsy at presentation, more became dialysis dependent at index visit discharge. When patients with >50% crescents in both the groups were compared similar results were seen except that infective complications and proliferative lesions were more in ICCGN. When patients with <50% crescents in both the groups were compared similar findings were seen except that no difference was seen in clinical features and dialysis dependency between them.Conclusions: Oliguria at presentation, Hb <9 g/dl, index visit eGFR<15 ml/min, crescents >60%, moderate to severe IFTA are the independent risk factors for dialysis dependency at index visit discharge.